Management of Neonates With Tetralogy of Fallot
Study Questions:
What are the differences in outcomes between a strategy of staged repair (SR) (initial palliation [IP] and subsequent complete repair [CR]) as compared with primary repair (PR) for infants with symptomatic tetralogy of Fallot (sTOF)?
Methods:
A multicenter retrospective cohort study was performed. The study population included consecutive neonates with sTOF who underwent IP or PR at ≤30 days of age between 2005 and 2017 at the nine centers participating in the Congenital Cardiac Research Collaborative. The primary outcome was death, with secondary outcomes including hospital and intensive care unit lengths of stay, duration of cardiopulmonary bypass, anesthesia, ventilation, inotrope use, as well as complication and reintervention rates. Outcomes were compared using propensity-score adjustment.
Results:
A total of 572 patients were studied, of which 343 patients underwent SR and 230 underwent PR. For patients undergoing SR, the initial procedure was surgical in 256 and transcatheter in 86. Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the SR group (p ≤ 0.01). The observed risk of death was not different between the groups (10.2% vs. 7.4%; p = 0.25) at a median of 4.3 years. After adjustment, the hazard of death remained similar between groups (hazard ratio, 0.82; 95% confidence interval, 0.49-1.38; p = 0.456), but it favored SR during early follow-up (<4 months; p = 0.041). Secondary outcomes favored the SR group in component analysis, whereas they largely favored PR in cumulative analysis. Reintervention risk was higher in the SR group (p = 0.002).
Conclusions:
The authors concluded that when adjusted for patient-related factors, early mortality and neonatal morbidity were lower in patients undergoing staged repair, while cumulative morbidity and reinterventions favored the primary repair group, suggesting potential benefits to each strategy.
Perspective:
The optimal treatment strategy for infants with TOF requiring intervention in the neonatal period remains controversial and can be center-dependent. This study adds to the fund of knowledge related to outcomes after staged palliation as compared with complete repair. Not surprisingly, there is not likely a single best pathway for all patients. Individual patient factors, as well as the relative strengths of individual centers, surgeons, and interventional cardiologists should play a role in the decision making. There will likely be further evolution of palliative strategies with the continued development of transcatheter techniques related to stenting of the patent ductus and right ventricular outflow tract. Finally, the study emphasizes the importance of looking beyond the operative and short-term mortality and taking into account the longer-term patient outcomes.